Cystic Fibrosis

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McLachlan G, Baker A, Tennant P, Gordon C, Vrettou C, Renwick L, Blundell R, Cheng SH, Scheule RK, Davies L, Painter H, Coles RL, Lawton AE, Marriott C, Gill DR, Hyde SC, Griesenbach U, Alton EW, Boyd AC, Porteous DJ, Collie DD. 2007. Optimizing aerosol gene delivery and expression in the ovine lung. Mol Ther 15:348-354.
Griesenbach U, Kitson C, Escudero Garcia S, Farley R, Singh C, Somerton L, Painter H, Smith RL, Gill DR, Hyde SC, Chow YH, Hu J, Gray M, Edbrooke M, Ogilvie V, MacGregor G, Scheule RK, Cheng SH, Caplen NJ, Alton EW. 2006. Inefficient cationic lipid-mediated siRNA and antisense oligonucleotide transfer to airway epithelial cells in vivo. Respir Res 7:26.

Hirth BH, Qiao S, Cuff LM, Cochran BM, Pregel MJ, Gregory JS, Sneddon SF, Kane JL, Jr. 2005. Discovery of 1,2,3,4-tetrahydroisoquinoline-3-carboxylic acid diamides that increase CFTR mediated chloride transport. Bioorg Med Chem Lett 15:2087-2091.

van Heeckeren AM, Scaria A, Schluchter MD, Ferkol TW, Wadsworth S, Davis PB. 2004. Delivery of CFTR by adenoviral vector to cystic fibrosis mouse lung in a model of chronic Pseudomonas aeruginosa lung infection. Am J Physiol Lung Cell Mol Physiol 286:L717-726.

van Heeckeren AM, Schluchter M, Xue L, Alvarez J, Freedman S, St George J, Davis PB. 2004. Nutritional effects on host response to lung infections with mucoid Pseudomonas aeruginosa in mice. Infect Immun 72:1479-1486.

Watson M, Cutting G, Desnick R, Driscoll D, Klinger K, Mennuti M, Palomaki G, Popovich B, Pratt V, Rohlfs E, Strom C, Richards C, Witt D, Grody W. 2004. Cystic fibrosis population carrier screening: 2004 revision of American College of Medical Genetics mutation panel. Genet Med 6:387-391.

Xiao YF, Ke Q, Chen Y, Gregory J, Hirth BH, Kane JL, Cuff L, Qiao S, Pregel M. 2003. Effects of Genz-97179 and Genz-98826 on chloride currents in CFT1 cells. In: J of Gen Physiol. p 25a.

Richards CS, Bradley LA, Amos J, Allitto B, Grody WW, Maddalena A, McGinnis MJ, Prior TW, Popovich BW, Watson MS, Palomaki GE. 2002. Standards and guidelines for CFTR mutation testing. Genet Med 4:379-391.

Rohlfs EM, Zhou Z, Sugarman EA, Heim RA, Pace RG, Knowles MR, Silverman LM, Allitto BA. 2002. The I148T CFTR allele occurs on multiple haplotypes: a complex allele is associated with cystic fibrosis. Genet Med 4:319-323.

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